Center for Bioinformatics
Oxidoreductases | Transferases | Hydrolases | Lyases | Isomerases | Ligases

Basic Information

Enzyme Number

2.1.4.1

Official Name

glycine amidinotransferase

Name from literature

arginine glycine transamidinase

Pathway from literature

creatine synthesis

Pathway from KEGG

Amino Acid Metabolism; Urea cycle and metabolism of amino groups; map00220

Amino Acid Metabolism; Glycine, serine and threonine metabolism; map00260

Amino Acid Metabolism; Arginine and proline metabolism; map00330

Organisms

Human (9606)

Genome localization

15q21.1[2628 ],

Comments

Canavanine can act instead of arginine.

Rate-limiting Description

"We administered intravenous arginine to six patients and six controls to study in vivo inhibition by high ornithine concentrations of arginine-glycine transamidinase, the rate-limiting enzyme in creatine synthesis." (7419715)

Regulatory Information

Regulatory type

Detail

feedback;

"The enzyme was competitively inhibited by ornithine, with a Ki of 0.253 mM. Kidney arginase was inhibited only slightly and non-competitively. Most important in this respect is the feedback repression of AGAT by creatine, the end-product of the pathway. " (7378422)

phosphorylation;

P50440:from_uniprot:417_Phosphothreonine

Gene ontology

Gene ontology

GO:0006601 (P) creatine biosynthetic process [P50440 ];
GO:0005758 (C) mitochondrial intermembrane space [P50440 ];
GO:0015068 (F) glycine amidinotransferase activity [P50440 ];
GO:0016813 (F) hydrolase activity, acting on carbon-nitrog... [P50440 ];
GO:0005743 (C) mitochondrial inner membrane [P50440 ];

Tissue expression

Tissue From HPRD

Leukocyte [03838 ];
Pancreas [03838 ];
Liver [03838 ];
Kidney [03838 ];

Tissue specificity

Kidney [P50440 ];

Subcellular localization

Localization

mitochondrion [P50440 ];

membrane [P50440 ];

cytoplasm [P50440 ];

Disease relevance

Disease

Defects in GATM are the cause of L-arginine:glycine amidinotransferase deficiency (AGAT deficiency) [MIM:602360]. AGAT deficiency is a defect in creatine metabolism leading to mental retardation [P50440 ];

Links

SwissProt

P50440

Entrez Gene

2628

HPRD

03838



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  Last Modified: 2009-03-24  
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